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Clinical Brief
Endoscopic Correction of Severe Laryngomalacia
C. Venkatakarthikeyan, Alok Thakar and Rakesh Lodha 1
Departments of Otorhinolaryngology and 1Pediatrics, All India Institute of Medical Sciences, Ansari Nagar,
New Delhi, India.
Abstract. Tracheostomy for management of severe laryngomalacia is associated with significant morbidity and mortality. Two
cases are reported wherein the laryngeal abnormality was corrected by ary-epiglottic fold incision and CO2 laser
supraglottoplasty. Stridor and respiratory obstruction were relieved and a long term tracheostomy avoided. Endoscopic
correction of laryngomalacia offers significant benefits over conventional treatment with tracheostomy in terms of decreased
morbidity and improved quality of life. [Indan J Pedlatr 2005; 72 (2) : 165-168] E-mail: [email protected].
Key words : Laryngomalacia; Tracheostomy; Morbidity and mortality; Endoscopic correction
Laryngomalacia is the most frequent cause (60 to 70%) of
stridor in both newborns and infants. 1 Approximately
90% of reported cases are mild 2and the symptoms resolve
spontaneously by the age of two years. In 7 to 12% of
children the condition is severe and potentially life
threatening dyspnea may occur. In addition, there may be
swallowing difficulties, failure to thrive, obstructive sleep
apnea, cor pulmonale, 3 and even s u d d e n death. The
severity of the above symptoms may make it necessary to
resort to tracheostomy till a spontaneous improvement
occurs. Tracheostomy in y o u n g children, however,
carries significant m o r b i d i t y and m o r t a l i t y . 4.5
T r a c h e o s t o m y tube obstruction a n d accidental
decannulation are common risks. The children are also
prone to recurrent lower respiratory tract infections,
subglottic stenosis, speech delay and a psychological
dependence on the tracheostomy. These make its removal
difficult even after improvement of the airway. The long
term mortality from a pediatric tracheostomy, as reported
from the US and Europe, ranges between 0.9% to 5%. 4~
Similar figures from India m a y well be higher. In
addition, a tracheostomised child requires constant
attention and supervision.
The above m e n t i o n e d problems associated w i t h
tracheostomy, underline the unsatisfactory nature of this
t r e a t m e n t o p t i o n and the necessity of an a l t e r n a t e
strategy. This report describes two young children with
severe l a r y n g o m a l a c i a w h e r e i n the u p p e r a i r w a y
obstruction has been treated by supraglottic surgery and
a long- term tracheostomy avoided.
CASE REPORT
Case 1
A 20-day-old well-thriving female child was referred to
Correspondence and Reprint requests : Dr. Alok Thakar, Assistant
Professor, Department of Otorhinolaryngology,All India Institute of
Medical Sciences, Ansari Nagar, New Delhi- 110029. Fax: 91-1126588663, 26588641
Indian Journal of Pediatrics, Volume 72--February, 2005
the o t o l a r y n g o l o g y service with inspiratory stridor
manifesting from the early neonatal period. The child was
born at full term by vaginal delivery at hospital.
On examination, the child had inspiratory stridor,
minimal suprasternal recession but no intercostal or
subcostal recession. The stridor was exacerbated by
crying, but was not significantly altered in the supine or
prone position. The child weighed 3.5 kg. The respiratory
rate was 42/min and heart rate 140/min. The oral cavity
and oropharynx were normal. Laryngeal examination by
a flexible nasopharyngoscope revealed the epiglottis to be
omega-shaped and folded posteriorly so as to cap the
anterior 2/3 ~dof the larynx. The arytenoids were tall and
loose mucosal folds were prolapsing inwards with each
inspiratory breath. The vocal folds were normal. These
findings were suggestive of laryngomalacia. As the child
did not have significant respiratory distress, it was
decided to observe the child. Twenty-five days later, the
child was brought to the clinic with worsening of stridor,
significant chest retractions and two episodes of cyanosis.
She had not been feeding well and weighed 2.45 kg. On
examination, she had severe inspiratory stridor with
suprasternal and intercostal retractions, a respiratory rate
of 5 8 / m i n and a heart rate of 160/min. Fibreoptic flexible
laryngoscopic (FOL) examination showed increased
supraglottic collapse, in a d d i t i o n to the findings at
previous visit. The child was immediately scheduled for a
rigid endoscopy and probable tracheostomy u n d e r
general anesthesia.
The initial direct laryngoscopy and bronchoscopy,
undertaken with the patient breathing spontaneously,
confirmed the diagnosis of laryngomalacia (Fig. la). This
also excluded other abnormalities in the tracheo-bronchial
tree, particularly vocal cord paralysis and interarytenoid
cleft. The child w a s then i n t u b a t e d and
microlaryngoscopy undertaken. The short aryepiglottic
folds (AE folds) were divided by a sharp scissors at a
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Venkatakarthikeyan et al
point approximately midway between the arytenoid and
the epiglottis. Following this incision, the epiglottis was
found to immediately spring forward and the laryngeal
inlet was rendered more visible and open.
The child was extubated after reversal of anesthesia.
The stridor was significantly reduced and endoscopic
e x a m i n a t i o n c o n f i r m e d decrease in the supraglottic
collapse. Post-operatively she was nursed in the pediatric
ICU w h e r e i n she r e c e i v e d antibiotics, n e b u l i z e d
b u d e s o n i d e , a d r e n a l i n e , and nasogastric feeds. The
stridor progressively decreased and subsided completely
by the 3~ post-operative day. Oral feeds were started on
the 6~ post-operative day and easily tolerated without
aspiration. She was then discharged. At three weeks
follow up, the child did not have stridor, was taking
breast feeds adequately, and had gained weight. A repeat
FOL evaluation (Fig. lb) demonstrated no supraglottic
4,
i : ?.
collapse. At one year follow up, she is completely free of
stridor.
Case 2
A 2-year-old male child presented with progressively
increasing stridor since the first month of life which was
now associated with respiratory distress. The child was
born at full term in a hospital following an uneventful
labor and was judged as normal at birth. The child had
n o t b e e n f e e d i n g well a n d h a d p o o r w e i g h t gain.
Examination revealed inspiratory stridor with
suprasternal recession, a respiratory rate of 40/min, and
a heart rate of 132/min. The oral cavity and oropharynx.
were normal. FOL examination revealed omega-shaped
epiglottis and prominent arytenoids with loose mucosal
folds. The loose supraglottic tissues obscured the glottis
preventing assessment of vocal cord mobility.
Significant respiratory distress and incomplete clinic
evaluation necessitated further endoscopic evaluation
u n d e r g e n e r a l anesthesia. The initial e v a l u a t i o n
undertaken without an endotracheal tube confirmed the
diagnosis of laryngomalacia (Fig 2a). The cords were
mobile, and the glottis, subglottis and trachea w e r e
normal. The endotracheal tube was then passed and
microlaryngoscopy undertaken; the short AE folds were
divided bilaterally. The child was extubated on the first
postoperative day. However, there was recurrence of
stridor and respiratory distress. Therefore, a tracheostomy
h a d to be u n d e r t a k e n . A r e p e a t FOL e v a l u a t i o n
undertaken after a few weeks indicated the presence of
p e r s i s t e n t supraglottic collapse c o n s e q u e n t to loose
supraglottic tissues.
Fig la. Preoperativephotograph of case 1 showing classicalfeatures
of laryngomalacia: a tubular larynx, tall anteriorly
prolapsing arytenoids, short AE folds and a posteriorly
placed omega shaped epiglottis.
Fig. 2a. The preoperative photograph of case 2 demonstrates the
markedly tall and anteriorly prolapsing arytenoids with
loose mucosa.
Fig lb. Postoperative photograpl~0f'casel demonstrates the incised
division in the left AE fold with a much improved laryngeal
inlet and a normally positioned arytenoid and epiglottis.
166
A second surgery was scheduled wherein a complete
supraglottoplasty was undertaken with a CO2 laser so as
to excise the loose mucosa at the base of the epiglottis, AE
folds a n d false cord. P o s t o p e r a t i v e p e r i o d was
Indian Journal of Pediatrics, Volume 72--February, 2005
Endoscopic Correlation of Severe Laryngomalacia
uneventful. The child was discharged with the tracheostomy, with detailed instructions to the parents regarding
the care. Subsequent review at eight weeks with FOL,
confirmed good healing, with a patent supraglottis
devoid of any loose prolapsing tissues (Fig. 2b). The child
was then successfully decannulated.
t~
Fig2b. Same case after AE fold i n c i s i o n a n d a laser
supraglottoplasty demostrating a much improved laryngeal
inlet.
DISCUSSION
Severe stridor secondary to laryngomalacia has ominous
implications and necessitates immediate diagnostic and
therapeutic measures. A definitive diagnosis is best
achieved by a rigid endoscopy under general anesthesia.
The conventional treatment to relieve the stridor is a
tracheostomy. U n f o r t u n a t e l y these have significant
morbidity and occasional mortality in infants and young
children. 4.s The two cases reported here represent the
current approach to the diagnosis and t r e a t m e n t of
pediatric stridor and laryngomalacia. Most cases of
laryngomalacia are self-limiting or m a y improve with
age, making observation an established modality. Rigid
endoscopy poses a risk of precipitating a tracheostomy in
patients wherein it could otherwise have been avoided.
Instrumentation in the airway may precipitate local
edema and laryngospasm, and also the general anesthetic
m a y cause respiratory depression. These changes can
push a mildly compromised but compensated airway
towards a situation wherein a tracheostomy may become
imperative.
In our current practice we use FOL evaluation in
outpatient clinic to select the cases wherein observation is
persisted with and further rigid endoscopy deferred. Such
an evaluation helps confirm supraglottic and glottic
abnormalities and also facilitates a dynamic evaluationthis being especially relevant for laryngomalacia and
vocal cord palsy. H o w e v e r , the e v a l u a t i o n of the
subglottis and trachea is inadequate. This is important
limitation as tracheobronchial anamolies are known to be
Indian Journal of Pediatrics, Volume 72--February, 2005
multiple in 17.5 % of cases. 6
Approximately 10% patients with laryngomalacia
require surgery to secure the airway 7 although figures
upto 22O/o8 have been reported. Firm criteria for surgical
i n t e r v e n t i o n are difficult to define. L a r y n g o s c o p i c
appearance by itself is not used as the sole criterion to
d e t e r m i n e the n e e d for s u r g e r y , t h o u g h c o m p l e t e
obstruction of laryngeal inlet by supraglottic collapse is
certainly significant. Other features which are specifically
relevant include respiratory distress, apneic attacks,
feeding difficulty, failure to thrive and cor pulmonale.
The conventional surgical option of a tracheostomy to
relieve the obstruction is unsatisfactory, because of the
reasons already cited. Techniques, which directly improve
the airway, such as, reported in this communication, are
preferable in terms of their immediate efficacy and the
lack of any significant long-term morbidity.
The exact pathophysiology of laryngomalacia remains
obscure. It is h o w e v e r established that it leads to a
dynamic supraglottic collapse in inspiration, and the
following three anatomical abnormalities have been
chiefly implicated.
a. short AE folds
b. a long curled epiglottis which prolapses posteriorly
c. bulky arytenoids with loose mucosa which prolapses
forward on inspiration.
These features may be seen in c o m b i n a t i o n or as
separate entities.
Endoscopic surgical correction of laryngomalacia seeks
to correct these anatomic anomalies. The most consistent
of these structural abnormalities is the short aryepiglottic
fold9. It is believed that the short AE folds are responsible
for tethering the epiglottis posteriorly. Incision of the folds
midway along its length serves to release the epiglottis
and allows it to move forward. Hasslinger, in 1928, is
credited with the first description of such a procedure,
and several authors (Table 1) have described it since
then2,9llSuch division of the AE folds alone, is reported to
be sufficient to relieve the airway obstruction in the vast
majority of cases (90.6%).12We, therefore relied on AE fold
division as the initial procedure in both our cases.
The a l t e r n a t e surgical o p t i o n described for
l a r y n g o m a l a c i a is s u p r a g l o t t o p l a s t y in w h i c h the
obstructing supraglottic tissue is trimmed either with
microlaryngeal instruments or vaporized with CO2 laser
under suspension laryngoscopy. 13This was required in
o n e of the two cases reported here, wherein simple
division of the AE folds proved inadequate.
Following such direct laryngeal manipulation, the
resultant local inflammatory response to the surgical
trauma may potentially cause transient compromise of
the airway. Local edema and laryngospasm are everpresent dangers. The success of the procedure lies in
limiting surgical t r a u m a a n d in the availability of
pediatric intensive care facilities in the p o s t - o p e r a t i v e
period. In our cases, while we selected to use conventional
instrumentation for the AE fold excision, a CO2 laser was
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Venkatakarthikeyan et al
used for m o r e extensive supraglottoplasty. The CO2 laser
allows for precise a n d atraumatic m u c o s a l s u r g e r y and
m i n i m i s e s s u r g i c a l a n d t h e r m a l injury to the adjacent
tissues.
Complications inherent to the p r o c e d u r e are minimal,
p r o v i d e d a d e q u a t e p r e c a u t i o n s a r e e x e r c i s e d . The
i m p r o v e m e n t in a i r w a y t o o k 2 to 3 d a y s to be fully
manifested. Presumably this was the result of local tissue
e d e m a c o n s e q u e n t to b o t h the a n a e s t h e s i a a n d the
surgical procedure. Also, AE fold incision alone p r o v e d
i n a d e q u a t e in the s e c o n d case a n d a s e c o n d s u r g i c a l
procedure with laser supraglottoplasty w a s called for.
O u r results with b o t h the cases h a v e b e e n extremely
gratifying, and the literature indicates that no further long
term recurrence or morbidity is to be expected. As far as
the review of literature from index journals is concerned,
this is the first report of its kind in our country. The major
benefit has been the avoidance of the potential morbidity
and mortality of repeated intubation or tracheostomy. The
relief in airway compromise has also helped in correcting
for feeding difficulties and in facilitating n o r m a l growth.
CONCLUSION
We w o u l d like to e m p h a s i z e the utility of an initial FOL
e x a m i n a t i o n in the o u t p a t i e n t setting for evaluation of
children w i t h stridor. E n d o s c o p i c correction of severe
laryngomalacia is efficacious a n d serves to significantly
limit the morbidity and mortality that is inherent with the
conventional treatment of tracheostomy. The success of
the t e c h n i q u e h i n g e s on l i m i t i n g s u r g i c a l i n j u r y b y
adequate instrumentation, and b y expert post-operative
i n t e n s i v e care in an i n t e n s i v e care unit. C l o s e liason
b e t w e e n the paediatric and o t o l a r y n g o l o g i c a l teams is
called for.
168
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Indian Journal of Pediatrics, Volume 72--February, 2005